The existence of acquired cholesteatoma has been recognized for more than three centuries; however, the nature of the disorder has yet to be determined. Without timely detection and intervention, cholesteatomas can become dangerously large and invade intratemporal structures, resulting in numerous intra- and extracranial complications. Due to its aggressive growth, invasive nature, and the potentially fatal consequences of intracranial complications, acquired cholesteatoma remains a cause of morbidity and death for those who lack access to advanced medical care. Currently, no viable nonsurgical therapies are available. Developing an effective management strategy for this disorder will require a comprehensive understanding of past progress and recent advances. This paper presents a brief review of background issues related to acquired middle ear cholesteatoma and deals with practical considerations regarding the history and etymology of the disorder. We also consider issues related to the classification, epidemiology, histopathology, clinical presentation, and complications of acquired cholesteatoma and examine current diagnosis and management strategies in detail.

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Objective: To analyze the long-term safety of mastoid obliteration with cartilage in children with suppurative cholesteatomatous ears. Methods: The medical records of children (≤18 years) with cholesteatomas after primary tympanomastoidectomies were performed with cartilage obliteration over a 30-year period (1982-2012) were analyzed. The recidivism rate was calculated using the Kaplan-Meier survival analysis. Potentially confounding factors of recidivism were entered into a Cox regression model as covariates for multivariate analysis. Results: Of the 150 cholesteatomatous ears in 146 children, there were 95 discharging ears (63%) in 94 children. Among the 95 discharging ears, tympanomastoidectomy was performed with cartilage obliteration (CO group) in 77 ears (81%) and without cartilage obliteration (WO group) in 18 ears (19%). The mean follow-up period was 12 years. Recidivism was observed in 16 ears in the CO group and 4 ears in the WO group. The 10-year cumulative recidivism rates were comparable between the CO and WO groups (19 vs. 25%, p = 0.762). Multivariate analysis confirmed that mastoid obliteration was not a negative predictor of recidivism (p = 0.760). Recidivism of cholesteatoma was detected within 6.5 years after surgery in the WO group and was found as late as 16.1 years after surgery in the CO group. Cartilage could be maintained in the cavity with limited resorption, preventing reretraction pockets and subsequent recidivism. Conclusion: This study provides evidence supporting the long-term safety, feasibility and effectiveness of mastoid cartilage obliteration for children with suppurative cholesteatomatous ears. Despite comparable recidivism rates between the groups, the potential for the delayed detection of recidivism with cavity obliteration may warrant long-term follow-up, with careful attention paid to the potential for recidivism during postoperative care in children. i 2014 S. Karger AG, Basel

The Kaplan-Meier survival analysis method should be used when discussing recidivism rates, because the number of censored cases inevitably increases with observation time. Due to the late occurrence of recidivism and because the recidivism rate increases as time goes on, children should be periodically followed up for as long as possible.

Objective: Following cholesteatoma surgery, effective long-term hearing preservation in children is difficult and is not typically expected. Hence, long-term data on hearing outcomes are lacking. The aim of this study was to analyze long-term hearing outcomes in children following cholesteatoma surgery. Methods: For this study, 49 ears in 47 children (≤16 years) with acquired cholesteatomas following atticotomy-limited mastoidectomy with cartilage reconstruction (inside-out approach) during 1986–2010 were included. Pre- and post-operative recidivism-free audiometric results were compared. Hearing success was defined as a post-operative air conduction (AC) threshold of ≤30 dB (serviceable hearing). Logistic regression analyses were used to evaluate potential prognostic factors that independently contributed to the prediction of hearing success. These factors included stapes condition, pre-operative AC threshold, ossicular chain integrity, disease severity, age, and gender. Results: The mean duration of follow-up was 14.2 years. The post-operative AC (33.55 ± 15.42 dB) and air-bone gap (17.88 ± 12.94 dB) were significantly improved compared with the pre-operative AC (42.90 ± 16.47 dB, p < 0.001) and air-bone gap (30.23 ± 13.68 dB, p < 0.001). The probability of hearing success following surgery (40.8%) was significantly higher than prior to surgery (24.5%, p = 0.008). Multivariate logistic regression analyses revealed a statistically significant correlation between hearing success and stapes integrity only (p = 0.005). Conclusions: This study provides important information on effective long-term hearing preservation over a mean follow-up of 14 years. In addition, stapes destruction is an independent negative prognostic determinant of achieving hearing success. The prediction model in this study provides otologists with useful pre-operative information to inform patients and parents on expected hearing outcomes and may be useful for post-operative observations.